| • | a condition of painful red extremities in which a sensation of burning is associated with increased temperature |
| • | the principle feature of all types is that attacks are precipitated by exercise or heat, such as a warm bed |
| • | attacks last from a few minutes to several hours |
| • | labs: the most important association is with myeloproliferative disorders e.g. polycythemia vera or thrombocythemia (so check a CBC) |
| • | pathogenesis: myelproliferative vs. PVD (check pulses) vs. idiopathic (often congenital and familial - take a history) vs. drug (nifedipine, nicardipine) |
type I:
| • | associated with thrombocythemia |
| • | seen in polycythemia and myelofibrosis |
| • | hands and feet are usually involved |
type II: (primary/idiopathic)
| • | usually congenital, often familial |
| • | shares some similarities to reflex sympathetic dystrophy |
| • | provoked by exercise or exposure to warmth, occurring about a “critical point” which normally lies between 32° and 36°C and which is constant for each individual |
type III: (secondary erythromelagia)
| • | secondary to peripheral vascular disease (PVD) |
| • | one must understand that PVD is often characterized by high resting flows and vasodilatation of the microvascular bed. There is usually failure to respond to a further increase in metabolic demand, such as reflex hyperemia, or the needs of tissue repair following injury or ischemic necrosis |
| • | hands and feet usually involved |
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